A comparative study of the prognosis for Japanese patients with idiopathic interstitial pneumonia or BOOP based on histopathologic subsets]

We explored the prognosis for 123 patients with either idiopathic interstitial pneumonia (IIP) or bronchiolitis obliterans organizing pneumonia (BOOP). All patients underwent either open lung biopsy or thoracoscopic lung biopsy procedures. The histopathologic diagnosis of IIP included patients with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), and desquamative interstitial pneumonia with respiratory bronchiolitis-associated interstitial lung disease. The prognosis was poorest for patients with a histologic diagnosis of UIP, and excellent for those who received a diagnosis of BOOP. Although the prognosis is generally considered to be good for patients with NSIP, some NSIP patients in our study died. Histopathologic diagnosis based on surgical lung biopsy is useful in evaluating the prognosis for patients with IIP.     

Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes

BACKGROUND: To investigate whether the better prognosis of interstitial pneumonias associated with collagen vascular disease (CVD) compared with idiopathic interstitial pneumonia (IIP) is due to higher frequency of the nonspecific interstitial pneumonia (NSIP) pattern in CVD, we compared the outcomes of patients from these two groups with the same histopathologic pattern. SUBJECTS: The clinical features and survival of 362 patients (269 with IIP and 93 with CVD) diagnosed using surgical lung biopsy were analyzed. RESULTS: The mean survival of the CVD group (131.0 mo) was longer than that of the IIP group (80.5 mo) (p<0.0001). The patients with usual interstitial pneumonia pattern among the CVD group (n=36) was younger, female, and predominantly nonsmoking compared with the IIP group (n=203). Although baseline lung functions were not significantly different, the CVD group survived longer (mean, 177.0 mo) than the IIP group (mean, 66.9 +/- 6.5 mo; p=0.001). By multivariate analysis, younger age, better pulmonary function, and the presence of a CVD were independent prognostic factors. In NSIP pattern, no significant differences in survival, clinical features, or lung function were found between the two groups. CONCLUSION: Our data suggest that the better prognosis of patients in the CVD group is not solely due to the predominance of the NSIP pattern. The prognosis of patients with the usual interstitial pneumonia pattern in CVD is better than in those with idiopathic pulmonary fibrosis, despite the same pathologic pattern. In contrast, in those with an NSIP pattern, the prognosis is similar in both groups.     

Cyclosporin A treatment of interstitial pneumonia]

To assist in making prognoses for patients with interstitial pneumonia (IP) who were treated with cyclosporin A (CsA), we conducted a review of forty-nine patients (32 men and 17 women with a mean age of 62 yrs) with progressive IP during the period from 1997 through 2001. All patients were steroid-resistant or acutely exacerbated cases. They received a low dosage of CsA (100-130 mg/day) combined with corticosteroids. Before and after the CsA therapy, blood gas analysis and HRCT scans were evaluated. Twenty-five patients underwent video-assisted thoracoscopic surgery (VATS) or autopsy for a histopathological evaluation. Among the 49 patients with IP, the documented underlying systemic diseases were of unknown etiology (IPF or IIP) in 26 and were collagen vascular diseases (CVD) in 23. The chest CT pattern and underlying systemic diseases of IP were classified as a usual interstitial pneumonia (UIP) pattern/IPF in 16 cases, a non-UIP pattern/IIP in 10 cases, a UIP pattern/CVD in 7 cases, and a non-UIP pattern/CVD in 16 cases. The prognoses after CsA treatment were improved or unchanged in 27% of cases with a UIP pattern/IPF, 78% of cases with a non-UIP pattern/IIP, 71% of cases with a UIP pattern/CVD and 75% of cases with a non-UIP pattern/CVD; deteriorated in 73%, 22%, 29% and 25% of cases, respectively, with these patterns and underlying diseases. At present, four out of thirteen (31%) patients with acute exacerbation of UIP pattern/IPF have survived for four to twelve months (mean: 7.5 months). Four patients revealed re-exacerbation of IP after the dose of CsA was tapered. Among the 25 patients with IP, the histopathological patterns of IP were classified as usual interstitial pneumonia (UIP) in 10 cases, nonspecific interstitial pneumonia (NSIP) in 14 cases (group I, 2; group II, 5; group III, 7) and diffuse alveolar damage (DAD) in 1 case. The prognoses were improved or unchanged in all cases of NSIP group I, in 80% of cases with NSIP group II, in 29% of cases of NSIP group III and in 20% of cases of UIP; and deteriorated in the case of DAD, in 80% of cases of UIP, in 71% of cases of NSIP group III, and in 20% of cases of NSIP group II. It should be emphasized that CsA combined with corticosteroids may be effective for the treatment of steroid-resistant or acute exacerbation cases of IP. Further studies are required to determine long-term outcome with this treatment.     

A recurrent case of idiopathic interstitial pneumonia histologically diagnosed as nonspecific interstitial pneumonia group 1]

A 59-year-old man was admitted to our hospital because of dyspnea. Chest radiography showed infiltration and consolidation in both lung fields. He was clinically diagnosed as having idiopathic interstitial pneumonia (IIP), and histological examination of a thoracoscopic lung biopsy specimen showed nonspecific interstitial pneumonia (NSIP) group 1. Corticosteroid therapy had previously been effective, but about 1 year later the disease recurred. The patient's clinical condition was very similar to the first episode, and is improving in response to the same treatment again. Although it is generally accepted that patients with IIP diagnosed histologically as NSIP have a good prognosis, it should be remembered that recurrence is possible, even in patients with group 1.     

Idiopathic nonspecific interstitial pneumonia: lung manifestation of undifferentiated connective tissue disease?

RATIONALE: The American Thoracic Society/European Respiratory Society International Consensus Classification panel identified the clinical entity idiopathic nonspecific interstitial pneumonia (NSIP) as a provisional diagnosis and recommended further study. OBJECTIVES: We hypothesized that idiopathic NSIP is an autoimmune disease and the lung manifestation of undifferentiated connective tissue disease (UCTD), a recently described, distinct entity. METHODS: We studied 28 consecutive patients with idiopathic interstitial pneumonia (IIP) enrolled in the University of California, San Francisco Interstitial Lung Disease Center who met prespecified criteria for UCTD, as follows: at least one clinical manifestation of connective tissue disease, serologic evidence of systemic inflammation in the absence of clinical infection, and absence of sufficient American College of Rheumatology criteria for another connective tissue disease. Medical record reviews, evaluation of radiographs, and scoring of lung biopsies were performed. The control group consisted of all other patients (n = 47) with IIP who did not meet the UCTD criteria. MEASUREMENTS AND MAIN RESULTS: The patients with UCTD were more likely to be women, younger, and nonsmokers than the IIP control subjects. Compared with the control group, patients with UCTD-ILD were significantly more likely to have ground-glass opacity on high-resolution computed tomography (HRCT) and NSIP pattern on biopsy, and less likely to have honeycombing on HRCT or usual interstitial pneumonia on biopsy. At our center, the majority of patients classified as idiopathic NSIP (88%) met the criteria for UCTD. CONCLUSIONS: Most patients diagnosed with idiopathic NSIP meet the case definition of UCTD. Furthermore, these results show that the clinical entity idiopathic NSIP is different from idiopathic pulmonary fibrosis and appears to be an autoimmune disease.     

Nonspecific interstitial pneumonia: a real clinical entity?

Based on the current multidisciplinary classification of idiopathic interstitial pneumonia (IIP) organized by ATS/ERS, nonspecific interstitial pneumonia (NSIP) is considered as one type of IIP. An incidence of idiopathic NSIP is relatively small and possesses clinical features that are different than idiopathic pulmonary fibrosis (IPF) and usual interstitial pneumonia (UIP). Because there is little evidence of a long-term prognosis in patients with NSIP, some of them have an unfavorable prognosis similar to IPF/UIP. We review the significance of prognostic factors that have been reported in patients with IPF/UIP by applying them to patients with NSIP. The association with collagen vascular diseases focuses on etiologic background. Finally, the article discusses whether NSIP could be an early lesion of UIP based on the reported evidence and our own professional experiences.     

A case of idiopathic interstitial pneumonia with cytomegalovirus infection]

A 67-year-old female was admitted to our hospital because of fever, dry cough, and exertional dyspnea. The findings of chest X-ray, transbronchial lung biopsy, and bronchoalveolar lavage were compatible with the diagnosis of idiopathic interstitial pneumonia. Prednisolone was administered and she felt better for a while. However, she developed severe dyspnea, and marked diffuse infiltrative shadows were observed on chest X-ray after 3 months of steroid therapy. In spite of pulse therapy with methylprednisolone, she died of severe respiratory failure. Complement fixation test and IgG antibody enzyme immunoassay for cytomegalovirus were positive, but there was no change the titers between admission and death. IgM antibody was negative. The lung findings at autopsy compatible with usual interstitial pneumonia and diffuse alveolar damage, moreover, cytomegalovirus infection was observed. We consider that recurrent cytomegalovirus pneumonia had been present due to secondary immunodeficiency caused by administration of steroid hormones.     

Successful treatment using cyclosporine A plus corticosteroid therapy in an elderly patient with severe idiopathic interstitial pneumonia

An 81-year-old woman was referred to our hospital due to acute progressive respiratory failure. Her chest X-ray film showed bilateral interstitial changes and computed tomography revealed a diffuse ground-glass appearance. Histological examination of transbronchial lung biopsy specimens did not provide a final diagnosis. The patient was diagnosed as having idiopathic interstitial pneumonia (IIP) and was treated with corticosteroid therapy. The chest X-ray appearance improved temporarily after corticosteroid therapy, but the interstitial changes did not resolve and subsequently became worse again, so administration of cyclosporine A was added. After commencement of cyclosporine A, corticosteroid therapy could be gradually tapered over 10 months. This case suggests that a combination of steroid therapy with cyclosporine A is effective for severe IIP of unknown pathological diagnosis.     

非特异性间质性肺炎

非特异性间质性肺炎,是特发性间质性肺炎中继特发性肺间质纤维化后最常见的类型,它在流行病学、发病机制、临床表现、影像学特点、病理表现及预后等方面都有其自身特点,尤其是预后远较特发性肺间质纤维化好。本文将就目前对于NSIP的认识做一综述。     

High-BAL fluid concentrations of RANTES in nonspecific interstitial pneumonia compared with usual interstitial pneumonia

Chemokines such as regulated on activation, normal T-cell expressed and secreted (RANTES), monocyte chemoattractant protein (MCP)-1, monocyte inflammatory protein (MIP)-lalpha have been reported to play an important role in the pathogenesis of interstitial lung diseases. Among idiopathic interstitial pneumonia (IIP), nonspecific interstitial pneumonia (NSIP) has elevated percentages of Lymphocytes in bronchoalveolar lavage (BAL) fluid compared with usual interstitial pneumonia (UIP). These chemokines are candidate mediators for lymphocyte attraction to the lung in NSIR Therefore, we measured the BAL fluid levels of RANTES, MCP-1 and MIP1-alpha in 15 patients with idiopathic NSIP, 20 with idiopathic UIP, 22 with sarcoidosis and 12 healthy volunteers to evaluate the contribution of these chemokines using enzyme-linked immunosorbent assays. The levels of RANTES in BAL fluid were significantly higher in patients with NSIP compared with healthy volunteers (P < 0.01), UIP and sarcoidosis (P < 0.05). In MCP-1, the levels in BAL fluid of NSIP and UIP patients were significantly elevated compared with healthy volunteers and sarcoidosis patients (P < 0.01). These results suggest that RANTES and MCP-1 in BAL fluid may play an important role in inflammatory cell recruitment to the lung in idiopathic NSIP as well as other interstitial lung diseases.     

BAL findings in idiopathic nonspecific interstitial pneumonia and usual interstitial pneumonia.

Idiopathic pulmonary fibrosis (IPF), which has the histological pattern of usual interstitial pneumonia (UIP), is a progressive interstitial lung disease with a poor prognosis. Idiopathic interstitial pneumonias with a histological pattern of nonspecific interstitial pneumonia (NSIP) have a better prognosis than UIP, and may present with a clinical picture identical to IPF. The authors hypothesised that bronchoalveolar lavage (BAL) findings may distinguish between UIP and NSIP, and have prognostic value within disease subgroups. BAL findings were studied retrospectively in 54 patients with histologically proven (surgical biopsy) idiopathic UIP (n=35) or fibrotic NSIP (n=19), all presenting clinically as IPF. These findings were also compared with the BAL profile of patients with other categories of idiopathic interstitial pneumonias. BAL total and differential cell counts did not differ between the two groups. Survival was better in NSIP. In neither group were BAL findings predictive of survival or changes in lung function at 1 yr, even after adjustment for disease severity, smoking and treatment. BAL differential counts in fibrotic NSIP differed from respiratory bronchiolitis-associated interstitial lung disease, but not from desquamative interstitial pneumonia or cellular NSIP. The authors conclude that bronchoalveolar lavage findings do not discriminate between usual interstitial pneumonia and nonspecific interstitial pneumonia in patients presenting with clinical features of idiopathic pulmonary fibrosis, and have no prognostic value, once the distinction between the two has been made histologically.     

特发性间质性肺炎诊断中支气管镜的应用

目的分析评价支气管镜活检(TBLB)在特发性间质性肺炎(IIP)诊断中的应用价值。方法分析46例IIP患者的临床资料,结合其已有的胸部HRCT结果进行重新读片,并对于其相应的TBLB标本病理进行重新诊断,结合治疗效果,对比分析HRCT和TBLB的误诊和漏诊率;同时分析影响TBLB诊断价值的因素。结果对于UIP的诊断,HRCT的漏诊率为20%,误诊率为38.89%;TBLB的漏诊率为14.29%,误诊率为9.52%。结论 TBLB的漏诊率和误诊率显著低于HRCT;且当肺组织块数量≥3块时,TBLB具有较高的诊断价值;HRCT表现为非UIP者比HRCT表现为UIP更易获得有诊断价值的TBLB标本。     

The appearance of S-100 protein-positive dendritic cells and the distribution of lymphocyte subsets in idiopathic nonspecific interstitial pneumonia

Idiopathic interstitial pneumonia (IIP) is a progressive interstitial lung disease of unknown etiology. We investigated dendritic cells in idiopathic nonspecific interstitial pneumonia (NSIP) immunohistochemically, using anti-S-100 protein antibody and anti-HLA-DR antibody and also evaluated the relationship between the distribution of S-100 protein-positive dendritic cells (S- 100 DCs) and the lymphocytic subsets in the lung tissue of NSIP. Fifteen patients with the pathological diagnosis of idiopathic NSIP and six patients with usual interstitial pneumonia (UIP) were recruited into this study. Many S-100 DCs were observed in all the cases of idiopathic NSIP but S-100 DCs were not recognized in UIP cases invariably. In the mirror section method, most S-100 DCs showed a positive reaction of anti-HLA-DR antibody but a negative reaction for anti-CD1a antibody. CD8 and CD4 positive lymphocytes were infiltrated diffusely around S-100 DCs. It was demonstrated that the infiltration of CD8 positive lymphocytes predominated in the fibrosing areas and lymphoid follicles around S-100 DCs more so than CD4 positive lymphocytes.We speculate that the pathogenesis of NSIP is different from UIP and that DC and T cell-mediated immune mechanisms may play a role in the development and perpetuation of NSIP.     

Comparison of bronchoalveolar lavage fluids from nonspecific interstitial pneumonia and from ordinary interstitial pneumonia]

We studied cell findings in the bronchoalveolar lavage fluid (BALF) of 13 patients with nonspecific interstitial pneumonia (NSIP) and 20 with ordinary interstitial pneumonia (UIP). NSIP and UIP were difficult to distinguish by high-resolution CT. Surgical lung biopsies were performed in all patients. We divided the patients with NSIP and UIP into 4 groups, a group of idiopathic NSIP (idiopathic NSIP), a group of NSIP patients associated with collagen vascular disease (CVD NSIP), a group of idiopathic UIP patients (idiopathic UIP) and a group of UIP patients associated with collagen vascular disease (CVD UIP). We then examined the differences in BALF cell findings between these groups. The percentage of lymphocytes in BALF was higher in idiopathic NSIP and CVD NSIP than in the healthy control. The percentage of alveolar macrophages was lower and the percentage of lymphocytes was higher in CVD NSIP than in idiopathic UIP. The CD4/CD8 ratio in BALF of idiopathic NSIP was lower than with idiopathic UIP. It is important that NSIP be distinguished from UIP clinically, and our results suggest that BALF cell findings may be useful for making this distinction.     

Significance of connective tissue disease features in idiopathic interstitial pneumonia

In idiopathic interstitial pneumonia (IIP), the significance of connective tissue disease (CTD) features in the absence of a specific CTD diagnosis remains unclear. We studied the clinical and prognostic utility of a diagnosis of undifferentiated CTD (UCTD) in patients with biopsy-proven IIP. IIP patients undergoing surgical lung biopsy (1979-2005) were studied (nonspecific interstitial pneumonia (NSIP), n = 45; idiopathic pulmonary fibrosis, n = 56). UCTD was considered present when serum autoantibodies were present and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. A clinical algorithm that best predicted NSIP histology was constructed using a priori variables. The prognostic utility of UCTD, and of this algorithm, was evaluated. UCTD was present in 14 (31%) NSIP and seven (13%) IPF patients. UCTD was not associated with a survival benefit. The algorithm predictive of NSIP (OR 10.4, 95% CI 3.21-33.67; p<0.0001) consisted of the absence of typical high-resolution computed tomography (HRCT) features for IPF and 1) a compatible demographic profile (females aged <50 yrs) or 2) Raynaud's phenomenon. In patients with an HRCT scan not typical for IPF, this algorithm predicted improved survival (hazard ratio 0.35, 95% CI 0.14-0.85; p = 0.02) independent of IIP severity. UCTD is associated with NSIP histology. However, the diagnostic and prognostic significance of UCTD in IIP patients remains unclear.     

Nonspecific interstitial pneumonia (NSIP)

Nonspecific interstitial pneumonia (NSIP) represents one histologic subtype of idiopathic interstitial pneumonia (IIP). NSIP is typified by temporal homogeneity and less profusion of fibroblastic foci than is seen with usual interstitial pneumonia (UIP), the most common IIP. Clinically patients with NSIP present with similar symptoms (cough and dyspnea) when compared to patients with UIP. The duration of these symptoms prior to presentation is variable. The finding of fever may be more common in NSIP and clubbing may be more common in UIP; however, both findings can be seen in either UIP or NSIP. Physiological findings typically demonstrate a restrictive ventilatory defect with decreased gas transfer; little difference exists between UIP and NSIP. High resolution computed tomography (HRCT) scans are more likely to show honeycombing with UIP and a ground-glass pattern with NSIP, however, either of these findings can be seen with UIP or NSIP. The most striking differential feature between NSIP and UIP is the markedly better prognosis for patients with NSIP, a finding that cannot be explained by baseline differences in physiology or radiographic features. In this article we explore the clinical, physiological, and radiographic features of NSIP. We also review available information regarding response to therapy and prognosis.     

普通型间质性肺炎和非特异性间质性肺炎肺组织中不同细胞因子的表达及其意义

目的　研究转化生长因子 (TGF) β1、碱性成纤维细胞生长因子 (b FGF)、白细胞介素 8(IL 8)、白细胞介素 13(IL 13)、γ干扰素 (IFN γ)在普通型间质性肺炎 (UIP)和非特异性间质性肺炎(NSIP)肺组织中的分布、表达及意义。方法　经胸腔镜或开胸肺活检获取 5例UIP和 8例NSIP患者的肺组织。对照组 5例 ,来自手术切除的远离肺癌原发灶的周边肺组织。用免疫组化法半定量分析细胞因子的分布及表达。结果　TGF β1、IL 8、b FGF主要分布在肺泡上皮细胞、肺泡巨噬细胞、细支气管上皮细胞 ,UIP组表达强于NSIP组和对照组。IL 13主要分布在肺泡上皮细胞、肺泡巨噬细胞、间质单个核细胞 ,UIP、NSIP组表达无明显差异 ,但均强于对照组。IFN γ主要分布在间质单个核细胞 ,NSIP组表达强于UIP组和对照组。UIP组的IL 13/IFN γ比值为 (2 18± 0 76 ) ,NSIP组为(0 95± 0 2 8) ,对照组为 (0 91± 0 16 ) ,3组比较差异均有显著性 (P值均 <0 0 5 ) ,而NSIP组与对照组比较差异无显著性。对照组只有肺泡巨噬细胞表达上述各细胞因子。结论　TGF β1、IL 8、b FGF在UIP和NSIP患者肺组织中表达强度的不同和IL 13/IFN γ的是否平衡可能参与了UIP和NSIP不同的发病过程。     

Correlations of histologic classification with clinical features and prognosis in interstitial lung disease associated with collagen disease]

We studied 32 patients with interstitial pneumonia associated with collagen disease. All underwent surgical lung biopsy. Twenty-seven were histologically classified as follows: 8 with usual interstitial pneumonia (UIP), 10 with nonspecific interstitial pneumonia (NSIP), 6 with bronchiolitis obliterans organizing pneumonia (BOOP), and 3 with diffuse alveolar damage (DAD). Twenty-five of the patients were treated with corticosteroid but 4 (3 DAD, 1 NSIP) deteriorated rapidly and died within a month after lung biopsy. At the final follow-up, all 8 UIP patients were alive with residual respiratory impairment, whereas 6 NSIP and 4 BOOP patients had almost completely recovered. These findings suggested that histologic classification can be highly valuable to the therapeutic responsiveness and prognosis in cases of interstitial pneumonia associated with collagen disease.     

Unique characteristics of systemic sclerosis sine scleroderma-associated interstitial lung disease

STUDY OBJECTIVES: To describe the characteristics of systemic sclerosis sine scleroderma (ssSSc)-associated interstitial lung disease (ILD) presenting as idiopathic interstitial pneumonia (IIP). DESIGN: Retrospective review of six patients with ssSSc-associated ILD diagnosed after referral for evaluation of IIP. MEASUREMENT AND RESULTS: All patients were white, their mean age was 56 years (range, 37 to 86), and gender was evenly divided. Sclerodactyly, skin thickening, and digital edema were absent in all patients. All patients had scattered telangiectasia, and four patients had Raynaud phenomenon with abnormal nailfold capillaroscopy findings. All described gastroesophageal reflux, and three patients had esophageal dysmotility by esophagography. All had restrictive pulmonary physiology and a reduced diffusion capacity. High-resolution CT revealed nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP) radiographic patterns. Of the three patients who underwent surgical lung biopsy, two patients had NSIP and one patient had UIP pathologic patterns. Five patients had asymptomatic pericardial effusions and elevated pulmonary artery pressures by echocardiography. All patients had nucleolar-staining anti-nuclear antibodies (ANAs), and one patient was anti-Scl-70 positive. All five anti-Scl-70-negative patients were anti-Th/To positive, and the anti-Scl-70-positive patient was anti-Th/To negative. CONCLUSIONS: In the presentation of an IIP, the presence of a nucleolar-staining ANA, telangiectasia, Raynaud phenomenon with abnormal capillaroscopy findings, gastroesophageal reflux, or pericardial disease suggests underlying systemic sclerosis. These findings should aid clinicians in the evaluation and treatment of patients with otherwise undefined ILD.     

Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis

Background

Nonspecific interstitial pneumonia (NSIP) has recently been proposed as a histologic type of idiopathic interstitial pneumonia (IIP), but its broad spectrum of clinicopathologic findings and variable prognosis are poorly understood. It is particularly unclear how NSIP and usual interstitial pneumonia (UIP) are related. The present study investigated the clinicopathologic features and prognosis of NSIP, and its differential diagnosis from UIP.

Methods

The clinicopathologic findings and prognosis in 21 NSIP and 18 UIP patients who underwent surgical or video-assisted thoracoscopic lung biopsy were reviewed.

Results

NSIP was more frequent in women and showed nonspecific clinical manifestations. High-resolution computed tomography (HRCT) demonstrated ground-glass, net-like, and patchy attenuation in both lungs. Semiquantitative HRCT showed a median fibrosis score of 3 (range, 0 to 7) in NSIP patients and 5 (range, 2 to 7) in UIP patients (P<0.01). On histopathologic examination, NSIP cases were heterogeneous and the findings could be categorized into cellular and fibrosing patterns. The mean age of the NSIP and UIP patients was 48 and 60 years, respectively. The frequencies of fibroblast foci, myogelosis, honeycomb lesions, and pulmonary structural destruction in NSIP and UIP patients were 16.7% and 100% (P<0.001), 22.2% and 85.7% (P<0.05), 16.7% and 92.9% (P<0.001), and 27.8% and 100% (P<0.05), respectively. The responses to glucocorticoid treatment and the prognosis were significantly greater in NSIP than those in UIP.

Conclusions

NSIP was difficult to be differentiated from UIP by general clinical manifestations, but HRCT can be helpful for this purpose. Definitive diagnosis depends on the results of surgical lung biopsy.